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A neurofibrosarcoma is a rare kind of tumor. It is also sometimes called a peripheral nerve sheath tumor. This type of growth only develops in areas around the peripheral nerves, which are the parts of the nervous system that do not include the brain and the spinal cord. Examples include the nerves that run along the arms and legs.
Neurofibrosarcomas are classified as soft tissue sarcomas, which are cancers that begin in the body’s tissues. Unlike many kinds of cancers, a neurofibrosarcoma does not typically spread, or metastasize. When it does spread, it commonly attacks the lungs.
A patient may live with a neurofibrosarcoma for a long time before experiencing any symptoms. This is because it exists in soft tissues that can be pushed aside to make room for the growing tumor. Some common symptoms may include soreness and pain, which may be accompanied by a noticeable lump under the skin. Patients may also limp and have problems using the limbs normally.
It is unknown exactly what causes a neurofibrosarcoma to develop. They may occur due to genetic mutations. A person with this type of tumor may also have a higher risk of developing other kinds of cancers. Patients who suffer from Recklinghausen’s disease, also known as neurofibromatosis, are at a higher risk for neurofibrosarcomas. Neurofibromatosis is a genetic disease associated with a specific mutated gene and the development of other kinds of benign, or harmless, tumors.
A doctor who suspects that a patient may have a soft tissue sarcoma will often order x-rays and a magnetic resonance imaging (MRI) procedure. He may also request a computerized tomography (CT) scan. These diagnostic tests will allow the doctor to examine and evaluate the potential growth. Blood tests may also be required.
To confirm the diagnosis, a biopsy is often needed. This is a surgical procedure in which a small tissue sample of the neurofibrosarcoma is extracted. It can then be studied in a laboratory.
The recommended treatment for this kind of cancer often depends on how aggressive the tumor is, and whether it has spread, as well as the general health of the patient. Chemotherapy or radiation therapy may be recommended to shrink the tumors and destroy the cancer. These therapies may be used alone or together.
In some cases, the doctor may recommend surgery. The surgical removal of the tumor may necessitate the partial or complete removal of the affected limb. Sometimes, the limb may be saved through a process called limb-salvage surgery, or limb-sparing surgery. Patients who undergo this procedure, in which as much of the limb is preserved as possible, may need a full year of physical therapy to regain the full use of it. Other times, an amputation, or removal of the limb, is needed to preserve the patient’s life.
The general prognosis, or expected outcome, for a neurofibrosarcoma can vary widely from patient to patient. It will depend on the location and size of the growth, the general health of the patient, and the aggressiveness of the cancer. Patients who receive an early diagnosis and early treatment have a better chance of survival.
I am a survivor of Neurofibrosarcoma. I will be five years cancer free in February 2013.